Protein Name:
Trifunctional enzyme subunit alpha, mitochondrial
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UniprotKB/SwissProt ID: Q8BMS1 (Q8BMS1)
Gene Name:
Hadha
Organism: Mus musculus (Mouse)
Function: Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity. Independently of subunit beta, HADHA also exhibits a cardiolipin acyltransferase activity that participates in cardiolipin remodeling; cardiolipin is a major mitochondrial membrane phospholipid. HADHA may act downstream of Tafazzin/TAZ, that remodels monolysocardiolipin (MLCL) to a cardiolipin intermediate, and then HADHA may continue to remodel this species into mature tetralinoleoyl-cardiolipin. Has also been proposed to act directly on MLCL; capable of acylating MLCL using different acyl-CoA substrates, with highest activity for oleoyl-CoA
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion. Mitochondrion inner membrane
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Graphical Visualization of S-nitrosylation Sites:
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The S-nitrosylation sites of Q8BMS1
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
110 |
AGADINMLSS C TTPQEATRIS |
CCCHHHHHHH H HHHHHHHHHH |
4.4% |
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| 2 |
349 |
LMGLYNGQVL C KKNKFGAPQK |
CCCHHHHHHH H HHHHHHHHHH |
6.57% |
21278135
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| 3 |
470 |
KEVESVTPEH C IFASNTSALP |
CCCHHHHHHH H HHHHHHHHHH |
2.33% |
21278135
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| 4 |
550 |
KDGPGFYTTR C LAPMMSEVMR |
CCCHHHHHHH H HHHHHHHHHH |
3.4% |
21278135
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| 5 |
747 |
ESAYGTQFTP C QLLLDHANNS |
CCCHHHHHHH H HHHHHHHHHH |
3.96% |
21278135
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| 6 |
97 |
SAVLISSKPG C FVAGADINML |
CCCHHHHHHH H HHHHHHHHHH |
3.17% |
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